| Literature DB >> 15153218 |
Richard J Ing1, D Ryan Cook, Resai A Bengur, Eric A Williams, John Eck, Guy de L Dear, Allison K Ross, Frank H Kern, Priya S Kishnani.
Abstract
Pompe or Glycogen Storage Disease type II (GSD-II) is a genetic disorder affecting both cardiac and skeletal muscle. Historically, patients with the infantile form usually die within the first year of life due to cardiac and respiratory failure. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes and a resurgence of elective anaesthesia for these patients. Understanding the unique cardiac physiology in patients with GSD-II is essential to providing safe general anaesthesia.Entities:
Mesh：
Substances：
Year: 2004 PMID： 15153218 DOI： 10.1111/j.1460-9592.2004.01242.x
Source DB: PubMed Journal: Paediatr Anaesth ISSN： 1155-5645 Impact factor: 2.556